Macrophage Activation Syndrome in Children: Diagnosis and Management

被引：9

作者：

Bagri, Narendra Kumar ^{[1
]}

Gupta, Latika ^{[2
]}

Sen, Ethan S.

Ramanan, A. V.

机构：

[1] AIIMS, Dept Pediat, Div Pediat Rheumatol, New Delhi, India

[2] Sanjay Gandhi Postgrad Inst Med Sci, Dept Clin Immunol & Rheumatol, Lucknow, Uttar Pradesh, India

来源：

INDIAN PEDIATRICS | 2021年 / 58卷 / 12期

关键词：

Hyper-inflammation; Secondary hemophagocytic lymphohistiocytosis; Juvenile idiopathic arthritis; JUVENILE IDIOPATHIC ARTHRITIS; FERRITIN; THERAPY; EMAPALUMAB; FEATURES; DISEASE;

D O I：

10.1007/s13312-021-2399-8

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Macrophage activation syndrome is a severe yet under-recognized complication encountered in pediatric rheumatology. It manifests as secondary hemophagocytic lymphohistiocytosis leading to a hyper-inflammatory state resulting from an underlying cytokine storm. If unchecked, it may lead to multiorgan failure and mortality. Early diagnosis and timely initiation of specific therapy is pivotal for a successful outcome. This review outlines the key clinical and laboratory features and management of macrophage activation syndrome.

引用

页码：1155 / 1161

页数：7

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